特发性肺动脉高压合并系统性硬化症患者的功能分级Ⅱ型呼吸困难：症状轻微但结局严重。
Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome.
机构信息
Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, France.
出版信息
Rheumatology (Oxford). 2010 May;49(5):940-4. doi: 10.1093/rheumatology/kep449. Epub 2010 Feb 8.
OBJECTIVE
To describe the history of SSc-associated pulmonary arterial hypertension (SSc-PAH) in patients with New York Heart Association (NYHA) functional class (FC) II dyspnoea at diagnosis.
METHODS
Data at the time of diagnosis were collected and analysed retrospectively for 77 consecutive patients with SSc-PAH.
RESULTS
Twelve patients (15.6%) presented with PAH and NYHA FC II dyspnoea. After a mean follow-up of 44 months, only 4 out of the 12 PAH patients remained stable in FC II, while 8 showed worsening to FC III or IV. Three patients died during the observation period; two from PAH and one from rectal cancer. Survival in patients in FC II at diagnosis was 100, 91 and 80% at 1, 2 and 3 years, respectively.
CONCLUSIONS
A majority of patients with mildly symptomatic SSc-PAH in NYHA FC II at diagnosis have a severe disease with poor prognosis.
目的
描述在诊断时即出现纽约心脏协会（NYHA）功能分级（FC）II 级呼吸困难的系统性硬化症相关肺动脉高压（SSc-PAH）患者的历史。
方法
对 77 例连续 SSc-PAH 患者的诊断时数据进行了回顾性收集和分析。
结果
12 例（15.6%）患者出现肺动脉高压和 NYHA FC II 级呼吸困难。在平均随访 44 个月后，12 例肺动脉高压患者中仅有 4 例在 FC II 级保持稳定，而 8 例恶化至 FC III 级或 IV 级。在观察期间，有 3 例患者死亡，其中 2 例死于肺动脉高压，1 例死于直肠癌。在诊断时处于 FC II 级的患者的生存率分别为 100%、91%和 80%，在 1、2 和 3 年时。
结论
在 NYHA FC II 级诊断时出现轻度症状性 SSc-PAH 的大多数患者存在预后不良的严重疾病。
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